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a malignant tumor that rises from the children of this age, so the tumor is rarely
metanephric mesoderm cells of the upper discovered by this method.
pole of the kidney.
It accounts for 20% of solid tumors in Dx
childhood; there is no increased incidence A CT scan or UTZ== reveals the primary
based on sex or race. tumor and any points of metastasis.
Kidney function studies
glomerular filtration rate or blood
CAUSES urea nitrogen== to assess function
It occurs in association with congenital of the kidneys before surgery.
anomalies such as aniridia (lack of color
in the iris), cryptorchidism, hypospadias,
pseudohermaphroditism, cystic kidneys, MEDICAL MANAGEMENT
hemangioma, and talipes disorders. Nephroblastomas are staged to predict
A number of genes have been identifi ed therapy and prognosis.
as associated with the disorder, e deletion Nephrectomy (excision of the affected
of chromosome 11. kidney).
Without therapy, metastatic spread by the Followed immediately by radiation
bloodstream is most often to the lungs, therapy (omitted in stage I tumors) and by
regional lymph nodes, liver, bone, and, chemotherapy with
eventually, brain. dactinomycin, doxorubicin, or
vincristine, cyclophosphamide,
S/Sx and etoposide.
Usually discovered early in life Chemotherapy may be given at
(6 months to 5 years; peak at 3 to 4 years) varying intervals for as long as 15
although it apparently arises from an months.
embryonic structure present in the child A second surgical procedure may be
before birth scheduled after 2 or 3 months to remove
Nephroblastomas distort the kidney any remaining tumor.
anteriorly so that the tumor is felt as a If tumor involvement is bilateral, the
firm, nontender abdominal mass. Parents operative decisions obviously become
are aware their infant has a mass in the more complex.
abdomen but bring the infant to their If the tumors are small, both tumors may
health care provider thinking that it is hard be removed, leaving functioning kidney
stool from chronic constipation. on both sides intact.
Fathers may discover the tumor when they In other children, the kidney with the
toss a baby in the air, catch the infant by larger tumor is removed and the tumor
the abdomen, and feel the abdominal site in the remaining kidney is then
mass. treated with both radiation and
Parents often report that the mass seemed chemotherapy.
to appear overnight== This actually can
happen, because tumors can hemorrhage NX MNGT
into themselves, doubling their size in a
matter of hours. Note; Little time can be allotted for
accompanying signs may be preoperative testing, because these tumors
hematuria and a low-grade fever. metastasize rapidly as a result of the large
The child may be anemic from blood loss blood supply to the kidneys and adrenal
and lack of erythropoietin formation by glands.
the diseased kidney. It is important that the child’s abdomen
Although hypertension may also occur not be palpated any more than is necessary
because of excessive renin production, for diagnosis, because handling appears to
aid metastasis. Place a sign reading “No
Abdominal
Palpation” over the child’s crib to help
prevent this.
COMPLICATIONS
Nephritis
small bowel obstruction
hepatic damage caused by fi brotic
scarring from radiation can occur.
In girls, radiation-related damage to the
ovaries may result in sterility.
Radiation to the lungs may result in
interstitial pneumonia; spine radiation can
result in scoliosis.
PROGNOSIS
Overall therapy for nephroblastoma is so effective
that about 90% of children who had no metastatic
spread at diagnosis survive for at least 5 years.